Epidermal Necrolysis: 60 Years of Errors and Advances

Y.K. Heng; H.Y. Lee; J.-C. Roujeau

Disclosures

The British Journal of Dermatology. 2015;173(5):1250-1254. 

In This Article

Initial Report and a Brief History of Relevant Terminology

The term 'toxic epidermal necrolysis' was introduced by Dr Alan Lyell in 1956.[2] It was used to describe a condition involving extensive destruction of the epidermis that resembled second-degree burns, associated with severe constitutional symptoms. As later acknowledged by Dr Lyell himself,[3] the report consisting of four cases had inadvertently included two other conditions, namely staphylococcal scalded skin syndrome and generalized bullous fixed drug eruption. This demonstrates the difficulties in diagnosing TEN and distinguishing it from other clinically similar diseases. The main differential diagnoses for EN are listed in Table 1.

The terms erythema multiforme (EM) and SJS have been historically linked to TEN and are often confused by clinicians even today. Dr Ferdinand von Hebra gave the initial description of EM in 1866.[4] EM presents classically as a localized predominantly acral eruption of papules that evolve into targetoid lesions, with no or mild involvement of mucous membranes and minimal constitutional symptoms.

In 1922 Stevens and Johnson first described patients with fever, skin lesions that were somewhat EM-like, eruptive stomatitis and ophthalmic involvement.[5] The term SJS came into common use in medical literature in the 1940s and was erroneously classified as a severe form of EM. In the 1950s, it was proposed that the terms 'EM minor' be used for the mild cutaneous form as originally described by von Hebra, and 'EM major (EMM)' for cases with more severe mucocutaneous involvement, including SJS.[6] Progression of cases phenotypically from SJS to TEN was also observed and hence TEN was for years included in an 'EM spectrum' of disease.[7]

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